Cystic Fibrosis (CF) is an inherited disorder in which mucus-secreting glands in the lungs produce considerable quantity of thick, sticky secretions that clog the airways, promote bacterial growth and lead to chronic obstruction, inflammation and destruction of the airways.

The purpose of this study is to collect data about the resolution of the chronic inflammatory state in addition to assure the safety of the therapy in CF patients.

In CF patients the unregulated inflammatory response overwhelms the normal protease (elastase)/antiprotease (AAT) balance, leading to the accumulation of elastase in the lung, destruction of the lung architecture, severe pulmonary dysfunction, and ultimately death.

Administration of AAT is to address the elastase/antiprotease imbalance in order to prevent destruction of the lung tissue and reduce the inflammatory dysregulation that causes pulmonary dysfunction.

Steady disease state for 3 months and no decrease in lung function exceeding 10% during that period

Colonization

Stable concomitant therapy >2 weeks prior to visit 1 and during the study

Non-tobacco user of any kind

Ability for sputum induction

Written informed consent

Exclusion Criteria:

Severe CF with an FEV1 of <25% predicted

History of lung transplant

Active allergic bronchopulmonary aspergillosis (ABPA)

Treatment with additional antibiotics (beyond standard CF treatment) for a period of 14 days before study entry (routine antibiotics permitted)

Treatment with additional oral and/or IV steroids (beyond standard CF treatment) for a period of 14 days before study entry (screening day)

Known hypersensitivity to plasma products

IgA deficiency

Uncontrolled hypertension

Lung surgery in the previous two years

Being on any thoracic surgery waiting list

Severe concomitant disease

Hospitalization within 1 month before study entry, not due to an airway disease

Severe liver cirrhosis with ascites

Hypersplenism

Grade III/IV oesophageal varices

Active pulmonary exacerbation within the 4 weeks prior to screening

History of significant hemoptysis within the previous year

Use of tobacco products or recreational drugs

Pregnancy or breastfeeding

Any serious or active medical or psychiatric illness which, in the opinion of the investigator, would interfere with patient treatment, assessment, or compliance with the protocol.

Being a female of child-bearing age without adequate contraception

Participation in research study within 1 month

Contacts and Locations

Choosing to participate in a study is an important personal decision. Talk with your doctor and family members or friends about deciding to join a study.
To learn more about this study, you or your doctor may contact the study research staff using the Contacts provided below.
For general information, see Learn About Clinical Studies.

Please refer to this study by its ClinicalTrials.gov identifier: NCT00499837